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Sveen M, Thune JJ, Køber L, Vissing J. Cardiac Involvement in Patients With Limb-Girdle Muscular Dystrophy Type 2 and Becker Muscular Dystrophy. Arch Neurol. 2008;65(9):1196–1201. doi:10.1001/archneur.65.9.1196
To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD).
Neuromuscular Clinic and Department of Cardiology at Rigshospitalet.
One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis.
Main Outcome Measures
Clinical investigation, echocardiography, and electrocardiographic findings.
Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level of dystrophic changes on muscle biopsy.
This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.
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